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Protein

3-ketoacyl-CoA thiolase, peroxisomal

Gene

ACAA1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalytic activityi

Acyl-CoA + acetyl-CoA = CoA + 3-oxoacyl-CoA.

Pathwayi: fatty acid metabolism

This protein is involved in the pathway fatty acid metabolism, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway fatty acid metabolism and in Lipid metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei123Acyl-thioester intermediateBy similarity1
Active sitei377Proton acceptorPROSITE-ProRule annotation1
Active sitei408Proton acceptorPROSITE-ProRule annotation1

GO - Molecular functioni

  • acetate CoA-transferase activity Source: Reactome
  • acetyl-CoA C-acyltransferase activity Source: GO_Central
  • palmitoyl-CoA oxidase activity Source: UniProtKB

GO - Biological processi

  • alpha-linolenic acid metabolic process Source: Reactome
  • bile acid metabolic process Source: UniProtKB
  • fatty acid beta-oxidation Source: UniProtKB
  • fatty acid beta-oxidation using acyl-CoA oxidase Source: Reactome
  • very long-chain fatty acid metabolic process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Acyltransferase, Transferase

Keywords - Biological processi

Fatty acid metabolism, Lipid metabolism

Enzyme and pathway databases

BioCyciMetaCyc:HS00752-MONOMER.
ZFISH:HS00752-MONOMER.
ReactomeiR-HSA-2046106. alpha-linolenic acid (ALA) metabolism.
R-HSA-390247. Beta-oxidation of very long chain fatty acids.
R-HSA-6798695. Neutrophil degranulation.
UniPathwayiUPA00199.

Names & Taxonomyi

Protein namesi
Recommended name:
3-ketoacyl-CoA thiolase, peroxisomal (EC:2.3.1.16)
Alternative name(s):
Acetyl-CoA acyltransferase
Beta-ketothiolase
Peroxisomal 3-oxoacyl-CoA thiolase
Gene namesi
Name:ACAA1
Synonyms:ACAA, PTHIO
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

HGNCiHGNC:82. ACAA1.

Subcellular locationi

GO - Cellular componenti

  • intracellular membrane-bounded organelle Source: HPA
  • membrane Source: UniProtKB
  • peroxisomal matrix Source: Reactome
  • peroxisome Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Peroxisome

Pathology & Biotechi

Organism-specific databases

DisGeNETi30.
OpenTargetsiENSG00000060971.
PharmGKBiPA24419.

Chemistry databases

DrugBankiDB09069. Trimetazidine.

Polymorphism and mutation databases

BioMutaiACAA1.
DMDMi135751.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 26PeroxisomeCombined sourcesAdd BLAST26
ChainiPRO_000003406727 – 4243-ketoacyl-CoA thiolase, peroxisomalAdd BLAST398

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei59PhosphothreonineCombined sources1
Modified residuei60PhosphothreonineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP09110.
MaxQBiP09110.
PaxDbiP09110.
PeptideAtlasiP09110.
PRIDEiP09110.

2D gel databases

REPRODUCTION-2DPAGEIPI00012828.
UCD-2DPAGEP09110.

PTM databases

iPTMnetiP09110.
PhosphoSitePlusiP09110.
SwissPalmiP09110.

Expressioni

Inductioni

Peroxisomal thiolase is markedly induced (at the level of transcription) by various hypolipidemic compounds in parallel with the other two enzymes of the peroxisomal beta-oxidation system.

Gene expression databases

BgeeiENSG00000060971.
CleanExiHS_ACAA1.
ExpressionAtlasiP09110. baseline and differential.
GenevisibleiP09110. HS.

Organism-specific databases

HPAiHPA006764.
HPA007244.

Interactioni

Subunit structurei

Homodimer.1 Publication

Protein-protein interaction databases

BioGridi106548. 29 interactors.
IntActiP09110. 2 interactors.
MINTiMINT-3006670.
STRINGi9606.ENSP00000333664.

Structurei

Secondary structure

1424
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi38 – 45Combined sources8
Turni51 – 53Combined sources3
Turni55 – 58Combined sources4
Helixi61 – 76Combined sources16
Helixi80 – 82Combined sources3
Beta strandi86 – 89Combined sources4
Beta strandi91 – 94Combined sources4
Helixi95 – 97Combined sources3
Helixi98 – 107Combined sources10
Beta strandi116 – 120Combined sources5
Helixi122 – 124Combined sources3
Helixi125 – 138Combined sources14
Beta strandi143 – 152Combined sources10
Turni153 – 155Combined sources3
Helixi168 – 170Combined sources3
Helixi172 – 175Combined sources4
Helixi176 – 178Combined sources3
Helixi181 – 191Combined sources11
Helixi196 – 216Combined sources21
Turni217 – 222Combined sources6
Beta strandi226 – 231Combined sources6
Beta strandi237 – 242Combined sources6
Helixi254 – 259Combined sources6
Beta strandi263 – 265Combined sources3
Beta strandi279 – 289Combined sources11
Helixi290 – 296Combined sources7
Beta strandi302 – 311Combined sources10
Helixi314 – 319Combined sources6
Helixi320 – 332Combined sources13
Helixi336 – 338Combined sources3
Beta strandi339 – 344Combined sources6
Helixi349 – 359Combined sources11
Helixi363 – 365Combined sources3
Helixi372 – 375Combined sources4
Turni379 – 381Combined sources3
Helixi382 – 397Combined sources16
Beta strandi401 – 409Combined sources9
Turni410 – 412Combined sources3
Beta strandi413 – 421Combined sources9

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2IIKX-ray2.55A/B30-423[»]
ProteinModelPortaliP09110.
SMRiP09110.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP09110.

Family & Domainsi

Sequence similaritiesi

Belongs to the thiolase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1389. Eukaryota.
COG0183. LUCA.
GeneTreeiENSGT00760000119318.
HOGENOMiHOG000012239.
HOVERGENiHBG003112.
InParanoidiP09110.
KOiK07513.
OMAiPQGKEDG.
OrthoDBiEOG091G09C6.
PhylomeDBiP09110.
TreeFamiTF332308.

Family and domain databases

Gene3Di3.40.47.10. 4 hits.
InterProiIPR002155. Thiolase.
IPR016039. Thiolase-like.
IPR020615. Thiolase_acyl_enz_int_AS.
IPR020610. Thiolase_AS.
IPR020617. Thiolase_C.
IPR020613. Thiolase_CS.
IPR020616. Thiolase_N.
[Graphical view]
PfamiPF02803. Thiolase_C. 1 hit.
PF00108. Thiolase_N. 1 hit.
[Graphical view]
PIRSFiPIRSF000429. Ac-CoA_Ac_transf. 1 hit.
SUPFAMiSSF53901. SSF53901. 2 hits.
TIGRFAMsiTIGR01930. AcCoA-C-Actrans. 1 hit.
PROSITEiPS00098. THIOLASE_1. 1 hit.
PS00737. THIOLASE_2. 1 hit.
PS00099. THIOLASE_3. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P09110-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MQRLQVVLGH LRGPADSGWM PQAAPCLSGA PQASAADVVV VHGRRTAICR
60 70 80 90 100
AGRGGFKDTT PDELLSAVMT AVLKDVNLRP EQLGDICVGN VLQPGAGAIM
110 120 130 140 150
ARIAQFLSDI PETVPLSTVN RQCSSGLQAV ASIAGGIRNG SYDIGMACGV
160 170 180 190 200
ESMSLADRGN PGNITSRLME KEKARDCLIP MGITSENVAE RFGISREKQD
210 220 230 240 250
TFALASQQKA ARAQSKGCFQ AEIVPVTTTV HDDKGTKRSI TVTQDEGIRP
260 270 280 290 300
STTMEGLAKL KPAFKKDGST TAGNSSQVSD GAAAILLARR SKAEELGLPI
310 320 330 340 350
LGVLRSYAVV GVPPDIMGIG PAYAIPVALQ KAGLTVSDVD IFEINEAFAS
360 370 380 390 400
QAAYCVEKLR LPPEKVNPLG GAVALGHPLG CTGARQVITL LNELKRRGKR
410 420
AYGVVSMCIG TGMGAAAVFE YPGN
Length:424
Mass (Da):44,292
Last modified:October 1, 1989 - v2
Checksum:i71B2BBAFA06AE412
GO
Isoform 2 (identifier: P09110-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     149-181: Missing.
     272-331: Missing.

Note: No experimental confirmation available.
Show »
Length:331
Mass (Da):34,665
Checksum:i8137A0AC448FBA24
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_011904172E → D.Corresponds to variant rs156265dbSNPEnsembl.1
Natural variantiVAR_069148387V → A.1 PublicationCorresponds to variant rs2229528dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_046195149 – 181Missing in isoform 2. 1 PublicationAdd BLAST33
Alternative sequenceiVSP_046196272 – 331Missing in isoform 2. 1 PublicationAdd BLAST60

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X12966 mRNA. Translation: CAA31412.1.
X14813 mRNA. Translation: CAA32918.1.
X65140 Genomic DNA. Translation: CAA46270.1.
X65148 Genomic DNA. Translation: CAA46271.1.
AP006309 Genomic DNA. No translation available.
CH471055 Genomic DNA. Translation: EAW64516.1.
BC000635 mRNA. Translation: AAH00635.1.
BC011977 mRNA. Translation: AAH11977.1.
BC014474 mRNA. Translation: AAH14474.1.
CCDSiCCDS2673.1. [P09110-1]
CCDS46794.1. [P09110-2]
PIRiS17515. XUHUAB.
RefSeqiNP_001123882.1. NM_001130410.1. [P09110-2]
NP_001598.1. NM_001607.3. [P09110-1]
UniGeneiHs.643487.

Genome annotation databases

EnsembliENST00000301810; ENSP00000301810; ENSG00000060971. [P09110-2]
ENST00000333167; ENSP00000333664; ENSG00000060971. [P09110-1]
GeneIDi30.
KEGGihsa:30.
UCSCiuc003cht.4. human. [P09110-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X12966 mRNA. Translation: CAA31412.1.
X14813 mRNA. Translation: CAA32918.1.
X65140 Genomic DNA. Translation: CAA46270.1.
X65148 Genomic DNA. Translation: CAA46271.1.
AP006309 Genomic DNA. No translation available.
CH471055 Genomic DNA. Translation: EAW64516.1.
BC000635 mRNA. Translation: AAH00635.1.
BC011977 mRNA. Translation: AAH11977.1.
BC014474 mRNA. Translation: AAH14474.1.
CCDSiCCDS2673.1. [P09110-1]
CCDS46794.1. [P09110-2]
PIRiS17515. XUHUAB.
RefSeqiNP_001123882.1. NM_001130410.1. [P09110-2]
NP_001598.1. NM_001607.3. [P09110-1]
UniGeneiHs.643487.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2IIKX-ray2.55A/B30-423[»]
ProteinModelPortaliP09110.
SMRiP09110.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi106548. 29 interactors.
IntActiP09110. 2 interactors.
MINTiMINT-3006670.
STRINGi9606.ENSP00000333664.

Chemistry databases

DrugBankiDB09069. Trimetazidine.

PTM databases

iPTMnetiP09110.
PhosphoSitePlusiP09110.
SwissPalmiP09110.

Polymorphism and mutation databases

BioMutaiACAA1.
DMDMi135751.

2D gel databases

REPRODUCTION-2DPAGEIPI00012828.
UCD-2DPAGEP09110.

Proteomic databases

EPDiP09110.
MaxQBiP09110.
PaxDbiP09110.
PeptideAtlasiP09110.
PRIDEiP09110.

Protocols and materials databases

DNASUi30.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000301810; ENSP00000301810; ENSG00000060971. [P09110-2]
ENST00000333167; ENSP00000333664; ENSG00000060971. [P09110-1]
GeneIDi30.
KEGGihsa:30.
UCSCiuc003cht.4. human. [P09110-1]

Organism-specific databases

CTDi30.
DisGeNETi30.
GeneCardsiACAA1.
HGNCiHGNC:82. ACAA1.
HPAiHPA006764.
HPA007244.
MIMi604054. gene.
neXtProtiNX_P09110.
OpenTargetsiENSG00000060971.
PharmGKBiPA24419.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1389. Eukaryota.
COG0183. LUCA.
GeneTreeiENSGT00760000119318.
HOGENOMiHOG000012239.
HOVERGENiHBG003112.
InParanoidiP09110.
KOiK07513.
OMAiPQGKEDG.
OrthoDBiEOG091G09C6.
PhylomeDBiP09110.
TreeFamiTF332308.

Enzyme and pathway databases

UniPathwayiUPA00199.
BioCyciMetaCyc:HS00752-MONOMER.
ZFISH:HS00752-MONOMER.
ReactomeiR-HSA-2046106. alpha-linolenic acid (ALA) metabolism.
R-HSA-390247. Beta-oxidation of very long chain fatty acids.
R-HSA-6798695. Neutrophil degranulation.

Miscellaneous databases

ChiTaRSiACAA1. human.
EvolutionaryTraceiP09110.
GeneWikiiACAA1.
GenomeRNAii30.
PROiP09110.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000060971.
CleanExiHS_ACAA1.
ExpressionAtlasiP09110. baseline and differential.
GenevisibleiP09110. HS.

Family and domain databases

Gene3Di3.40.47.10. 4 hits.
InterProiIPR002155. Thiolase.
IPR016039. Thiolase-like.
IPR020615. Thiolase_acyl_enz_int_AS.
IPR020610. Thiolase_AS.
IPR020617. Thiolase_C.
IPR020613. Thiolase_CS.
IPR020616. Thiolase_N.
[Graphical view]
PfamiPF02803. Thiolase_C. 1 hit.
PF00108. Thiolase_N. 1 hit.
[Graphical view]
PIRSFiPIRSF000429. Ac-CoA_Ac_transf. 1 hit.
SUPFAMiSSF53901. SSF53901. 2 hits.
TIGRFAMsiTIGR01930. AcCoA-C-Actrans. 1 hit.
PROSITEiPS00098. THIOLASE_1. 1 hit.
PS00737. THIOLASE_2. 1 hit.
PS00099. THIOLASE_3. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiTHIK_HUMAN
AccessioniPrimary (citable) accession number: P09110
Secondary accession number(s): G5E935, Q96CA6
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 1, 1989
Last sequence update: October 1, 1989
Last modified: November 30, 2016
This is version 186 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 3
    Human chromosome 3: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.